Objective To study the correlation between two indicators of the intestinal barrier(i.e.,serum endotoxins and D-lac-tate)and enterogenic uremic toxins,including indoxyl sulfate(IS)and urea nitrogen,in patients with chronic kidney disease(CKD).Methods A total of 260 CKD patients were selected from outpatients and inpatients of the Department of Nephrology and Endocrinology,Wangjing Hospital,China Academy of Traditional Chinese Medicine,from April 2019 to August 2021,and were divided into the CKD stage 3 group(n=51),the CKD stage 4 group(n=130),and the CKD stage 5 group(n=79),according to CKD stage.Patients'age,gender,disease duration,and past medical history were recorded.Serum IS,urea nitrogen,serum endotoxin,D-lactate,and blood cre-atinine were detected.The estimated glomerular filtration rate(eGFR)was calculated.Patients'faeces were retained to detect the levels of Escherichia coli and Bifidobacterium bifidum colonies.The correlation between serum endotoxin and D-lactate and enterogenic uremic toxins was analyzed.Results Comparison of general data:There were no significant differences in age,gender,course of disease,previ-ous diabetes mellitus,and bifidobacteria levels between the three groups(P＞0.05),but there were significant differences in pre-exist-ing hypertension and coronary heart disease,IS,serum creatinine,urea nitrogen,eGFR,serum endotoxin,D-lactate acid,and E.coli(P＜0.05).Correlation analysis:Spearman analysis showed that serum endotoxin and D-lactate were significantly positively correlated with IS,urea nitrogen,and serum creatinine(P＜0.05),and negatively correlated with eGFR(P＜0.05).The results of Pearson analy-sis showed that serum endotoxin and D-lactate were significantly positively correlated with E.coli and Bifidobacterium(P＜0.05).Mul-tivariate linear regression analysis showed that IS was the independent influencing factor of serum endotoxin(P＜0.001)and IS and urea nitrogen were the independent influencing factors of D-lactate(P＜0.05).Conclusion Elevated serum endotoxin and D-lactate lev-els in patients with CKD are associated with enterogenic uremic toxins.

ObjectiveTo observe the clinical efficacy and safety of Chinese herbal enema combined with fumigation as adjunctive therapy for non-dialysis chronic kidney disease （CKD） patients of stage 3-5 with spleen-kidney yang deficiency and turbid toxin blood stasis pattern. MethodsA total of 120 non-dialysis CKD，patients of stage 3-5 with spleen-kidney yang deficiency and turbid toxin blood stasis pattern were randomly divided into treatment group and control group， with 60 cases in each group. The control group received conventional western medical treatment， while the treatment group additionally received Chinese herbal enema combined with fumigation， with enema and fumigation performed alternately， once every other day， three times a week. The treatment course for both groups was 4 weeks. The levels of serum creatinine （Scr）， estimated glomerular filtration rate （eGFR）， and the total score of traditional Chinese medicine symptoms were compared before and after treatment in both groups. The efficacy of traditional Chinese medicine symptoms and clinical efficacy were compared between the two groups after treatment. Adverse reactions in both groups were observed during the treatment period. ResultsThe total score of traditional Chinese medicine symptoms was significantly reduced after treatment in both groups （P＜0.01）. Compared to the control group after treatment， the treatment group showed significant decreases in Scr and the total score of traditional Chinese medicine symptoms， and a significant increase in eGFR （P＜0.05）. The total effective rate of traditional Chinese medicine symptoms in the treatment group （96.67%） was higher than that in the control group （46.67%， P＜0.01）， as well as the total effective rate of clinical efficacy in the treatment group （75.00%） versus that in the control group （28.33%， P＜0.01）. During the treatment period， the vital signs of patients in both groups remained stable， and there were no significant abnormalities in blood routine， urine routine， stool routine， liver function indicators， and electrocardiogram after treatment. ConclusionChinese herbal enema combined with fumigation as adjunctive therapy can significantly alleviate clinical symptoms， improve renal function， and demonstrate good safety for non-dialysis CKD patients of stage 3-5 with spleen-kidney yang deficiency and turbid toxin blood stasis pattern.

Edema is one of the typical symptoms of nephrotic syndrome， and symptomatic diuretics is mainly used in clinical practice but with unstable effect. This paper reported a case of refractory edema of nephrotic syndrome treated by acupuncture. The patient was mainly manifested as edema in both lower limbs， which were sunken when pressed and difficult to recover. After symptomatic treatment with western medicine， the effect was not obvious. In traditional Chinese medicine， this patient was diagnosed as spleen-kidney depletion and water-dampness internal accumulation syndrome. Based on the principle of “when swelling is below the waist， urination should be induced” in ZHANG Zhongjing's Essentials from the Golden Cabinet （《金匮要略》）， it is recommended to puncture at Shuidao （ST 28） and Shuifen （CV 9） to promote urination， at Sanyinjiao （SP 6）， Lougu （SP 7）， Yinlingquan （SP 9） and Zusanli （ST 36） to fortify the spleen and boost the kidneys and treat the root and branch simultaneously. After 3-day treatment， urine becomes smooth， and the edema of both lower limbs disappeared.

Objective We aimed to observe the effect of the traditional Chinese medicine(TCM)-based"tertiary hospital-community integrated"treatment program in patients with diabetic kidney disease.Methods A total of 126 patients from the Jiangtai and Cuigezhuang Communities in Chaoyang District were randomly divided into the experimental group and the control group(n=63 patients per group).In the experimental group,the"tertiary hospital-community integrated"treatment program was implemented(including TCM differentiated health preservation,chronic disease management,comprehensive diagnosis and treatment program of integrated Chinese and Western medicine),while in the control group,the existing chronic disease diagnosis,treatment,and management program in the community was implemented(including chronic disease management with regular follow-ups,diagnosis and treatment program of Western medicine).The observation period was 6 months,with 3 months as a course of treatment.The 24 h urine total protein level(24 hUTP),the serum level of creatinine(Scr),and the estimated glomerular filtration rate(eGFR)were compared between the two groups,as well as the effective rates of 24 hUTP,Scr,and eGFR,the rate of achieving standard glucose levels and normal lipid metabolism,including low-density lipoprotein-cholesterol(LDL-C),triglyceride(TG),and glycosylated hemoglobin(GHB),the level of patients'self-management,and the medical service in utilization.Results There were 120 patients included for analysis(60 in the experimental group and 60 in the control group).The difference in 24 hUTP was significantly different(P＜0.05),while Scr and eGFR were not statistically different between the experimental and control groups after 3 months of treatment.The differences in 24 hUTP,Scr,and eGFR were statistically significant after 6 months(P＜0.05).After 6 months of treatment in both groups,the effective rates of 24 hUTP,Scr,and eGFR were higher in the experimental group than in the control group(78.3％,48.3％,and 50.0％in the experimental group and 35.0％,18.3％,and 15.0％in the control group,respectively)(P＜0.05);after 6 months,the LDL-C,TG,and GHB qualified rates were higher in the experimental group than in the control group(75.0％,83.3％,and 71.7％in the experimental group and 56.7％,63.3％,and 46.7％in the control group,respectively;P＜0.05);comparing the self-management levels of the two groups after 3 and 6 months of treatment,the total self-management score and the total self-efficacy score were both higher in the experimental group than in the control group(P＜0.05);comparing the time of hospitalization and hospitalization costs of the two groups 6 months after enrollment,the time of hospitalization and hospitalization costs were lower in the experimental group(P＜0.05).Conclusion The"tertiary hospital-community integrated"TCM-based treatment program improves renal function,glucose and lipid metabolism,and patients'self-management;it can reduce the economic burden of families,save medical resources,and improve the utilization of medical services.

Lymphoma-associated hemophagocytic syndrome is aggressive with rapid progression, particularly in NK/T cell lymphoma. The MINE regimen is a salvage treatment for aggressive non-Hodgkin lymphoma. In our center, the modified MINE regimen was applied to treat three patients with hemophagocytic syndrome secondary to aggressive NK cell leukemia and T-cell lymphoma. The modified MINE regimen showed good efficacy against NK/T cell lymphoma, control of the inflammatory state of secondary hemophagocytic syndrome, and good tolerability.

ObjectiveTo observe the effectiveness and safety of Qihuang Yishen Granules （芪黄益肾颗粒） combined with traditional Chinese medicine （TCM） chronic disease management on patients with diabetic kidney disease with deficiency qi and yin. MethodsTotally 140 patients diagnosed as diabetic kidney diseases with deficiency of both qi and yin were randomly divided into control group and trial group，with 70 cases in each group. Patients in the control group were treated with symptomatic treatment and routine chronic disease management. Patients in the trial group added Qihuang Yishen Granules and chronic disease management with TCM characteristics on the basis of symptomatic treatment. The course of treatment in both groups lasted for 6 months. The changes of laboratory indexes and chronic disease management level scores of the two groups of patients before treatment and after 3 and 6 months of treatment were compared， and their correlation were analyzed.The laboratory indexes of urinary protein and renal function related indicators such as 24-hour urinary protein quantification （24 hUTP）， serum creatinine （Scr）， blood urea nitrogen （BUN）， and estimated glomerular filtration rate （eGFR）， and control compliance rate， as well as blood glucose and lipid related indicators such as glycosylated hemoglobin （HbA1c）， low-density lipoprotein cholesterol （LDL-C）， triglyceride （TG） and control compliance rate were observed. The chronic disease management level scores included exercise，cognitive symptom management practice，communication with doctors， self-efficacy of symptom management，self-efficacy of disease commonness management. ResultsFinally，67 cases in the control group and 68 cases in the trial group completed the study. Compared with the group before treatment， the trial group had lower 24 hUTP and Scr， higher exercise score， total self-management score and all self-efficacy scale scores， higher TG at 3 months of treatment； at 6 months of treatment， the trial group had lower 24 hUTP， higher eGFR， and higher self-management scores and self-efficacy scale scores of all chronic diseases （P＜0.05）， and the control group had higher self-management total score （P＜0.05）. After 6 months of treatment， the trial group had lower 24 hUTP， Scr， LDL-C， and TG， higher eGFR， higher compliance rate of 24 hUTP， eGFR， LDL-C， and TG， and higher scores for all chronic disease management indexes compared with the control group （P＜0.05）. In terms of the correlation between laboratory indicators and chronic disease management level scores：there was a statistically significant difference in the correlation between 24hUTP and exercise，symptom management self-efficacy，and self-efficacy of disease commonness management （P＜0.05 or P＜0.01），all of which were negatively correlated. There was a statistically significant difference in the correlation between Scr and symptom management self-efficacy and self-efficacy of disease commonness management （P＜0.01），both of which were negatively correlated. There was a statistically significant difference in the correlation between eGFR and symptom management self-efficacy and self-efficacy of disease commonness management （P＜0.01），both of which were positively correlated. ConclusionQihuang Yishen Granules combined with chronic disease management of TCM can improve the level of proteinuria，renal function and lipid metabolism in patients with diabetic kidney disease with deficiency of both qi and yin，thus delaying the progress of diabetic kidney disease and also improve the level of chronic disease management of patients.

Objective:To report a family of Zellweger spectrum disorders (ZSD) caused by mutations in the PEX10 gene and to increase the level of awareness of the disease among clinicians. Methods:The clinical and genetic data of a brother-sister co-morbidity family with ZSD due to PEX10 gene mutation admitted to the Department of Neurology, Qilu Hospital of Shandong University in July 2022 were collected and a literature review was performed. Results:The proband was a 24-year-old female who was admitted to the Qilu Hospital of Shandong University due to unstable walking for 8 years and aggravated for 1 year with squatting effort. Cranial magnetic resonance imaging (MRI) showed cerebellar atrophy, electromyography suggested peripheral neuropathy with motor and sensory involvement, and pure tone hearing threshold measurement showed neurogenic deafness. The 15-year-old brother of the proband presented with "unsteadiness in walking and difficulty in squatting for 2 years". His cranial MRI and electromyography were similar to those of the proband, and his laboratory results suggested abnormal liver function. The whole exon sequencing results of the proband suggested a compound heterozygous mutation in the PEX10 gene with c. 992G>A(p. R331Q) and c. 988T>C(p. C330R) mutations and both were likely pathogenic mutations and respectively from her parents. And her brother also carried the above variants. A total of 9 case reports in English literature (1 of which was published by domestic scholars) were retrieved from major domestic and international databases on PEX10 gene mutations causing ZSD, with a total of 15 patients, most of whom had childhood and adolescent onset. The most common initial symptom was slowly progressed ataxia. The majority of patients showed cerebellar atrophy on cranial MRI and peripheral neuropathy was found in the most patients.The 2 patients were suggested to have phytic acid-free food, at the same time, they bagan taking L-arginine and ursodeoxycholic acid. Fortunately,both the ataxia symptom and liver function of them were dramatically alleviated 3 months later. Conclusions:For the patients with unexplained ataxia with polyneuropathy and abnormal liver function,the possibility of ZSD should be considered. Phytic acid-free food, L-arginine and ursodeoxycholic acid supplement may be beneficial for the ZSD patients.

Cancer patients have a fear of cancer recurrence long after their initial diagnosis and treatment. Fear of cancer recurrence has an important impact on patients' confidence and adherence to treatment, quality of life, and social functioning, and is associated with prognosis. This article reviews the theoretical model, related factors and intervention strategies of fear of cancer recurrence, so as to provide a reference for clinical intervention to reduce the level of fear of cancer recurrence.

Objective:To analyze the clinical features, imaging findings and gene test of patients with type Ⅱ Alexander disease.Methods:All the clinical data of three cases with type Ⅱ Alexander disease from August 2018 to June 2020 in the Department of Neurology, Qilu Hospital of Shandong University (Qingdao) and Qilu Hospital of Shandong University were collected, and their clinical and imaging findings were analyzed retrospectively.Results:All the three patients were middle aged and old men with a chronic progressive course, beginning with weakness of one or both lower limbs, followed by dizziness, dysarthria, dysphagia, sphincteral disturbances, constipation and orthostatic hypotension. Three patients all experienced misdiagnosis (hydrarthrosis, cerebral vascular disease, alcoholism, respectively) at early stage of the disease. Cranial magnetic resonance imaging (MRI) showed mild supratentorial periventricular leukodystrophy, which was not specific. Sagittal cranial MRI demonstrated medulla oblongata and upper cervical cord atrophy called “tadpole atrophy”, which had high suggestive value. The results of gene analysis showed heterozygous mutation of glial fibrillary acidic protein gene, which had been reported as pathogenic gene; c.1091C>T (p.A364V) in exon 6, c.722C>T (p.R258C) in exon 4 and c.197G>A (p.R66Q) in exon 1, respectively.Conclusions:Type Ⅱ Alexander disease is an autosomal dominant disease, most with point mutations, rarely with deletion mutations. Type Ⅱ Alexander disease should be suspected when a patient had signs of lower brainstem involvement such as dizziness, ataxia, pyramidal sign, autonomic dysfunction, especially when cranial MRI showed mild supratentorial leukodystrophy, and medulla oblongata and upper cervical cord atrophy.

Objective:To summarize and analyze the clinical data of Chinese patients with colony-stimulating factor 1 receptor (CSF1R)-related leukoencephalopathy, and clarify the phenotypic and genetic characteristics of Chinese patients.Methods:Medical history of patients with CSF1R-related leukoencephalopathy diagnosed from April 1, 2018 to January 31, 2021 in the department of neurology of 22 hospitals in China was collected, and scores of Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment Scale (MoCA), magnetic resonance severity scale were evaluated. Group comparison was performed between male and female patients.Results:A total of 62 patients were included, and the male-female ratio was 1∶1.95. The age of onset was (40.35±8.42) years. Cognitive impairment (82.3%, 51/62) and motor symptoms (77.4%,48/62) were the most common symptoms. The MMSE and MoCA scores were 18.79±7.16 and 13.96±7.23, respectively, and the scores of two scales in male patients (22.06±5.31 and 18.08±5.60) were significantly higher than those in females (15.53±7.41 , t=2.954, P=0.006; 10.15±6.26, t=3.328 , P=0.003). The most common radiographic feature was bilateral asymmetric white matter changes (100.0%), and the magnetic resonance imaging severity scale score was 27.42±11.40, while the white matter lesion score of females (22.94±8.39) was significantly higher than that of males (17.62±8.74 , t=-2.221, P<0.05). A total of 36 CSF1R gene mutations were found in this study, among which c.2381T>C/p.I794T was the hotspot mutation that carried by 17.9% (10/56) of the probands. Conclusions:The core phenotypic characteristics of CSF1R-related leukoencephalopathy in China are progressive motor and cognitive impairment, with bilateral asymmetrical white matter changes. In addition, there exist gender differences clinically, with severer cognitive impairment and imaging changes in female patients. Thirty-six CSF1R gene mutations were found in this study, and c.2381T>C/p. I794T was the hotspot mutation.